Vaibhav Pandey

Propranolol for infantile haemangiomas: Experience from a tertiary center

Aim: Infantile haemangiomas are the most common tumor of infancy. We report the use of propranolol for treatment of problematic and complicated haemangiomas. Patients and Methods: Propranolol was given to 52 children with mean age of 18.2 months at onset of treatment. After clinical and electrocardiographic evaluations, propranolol was administered with a starting dose of 2 mg/kg per day, given in 3 divided doses. Monthly follow up was done, response to oral propranolol therapy and any complications of therapy were recorded. Response to propranolol was classified as Complete Response, Excellent Response, Partial Response and Non Responder. Results: Total 49 patients showed significant improvement after propranolol therapy out of which 4 patients were complete responder, 30 patients (56.7%) were excellent responders; 15 patients (28.8%) were partial responders. 3 patients (5.7%) had growth of haemangiomas despite propranolol therapy and were classified as non-responder. Side effect like hypotension, rashes, gastroesophageal reflux was reported by 3 patients. In our study mean duration of treatment was 6.5 months. At the end of treatment propranolol was stopped by with gradual tapering of dose over a period of 2 weeks. Conclusion: Propranolol administered orally at 2 mg/kg per day has rapid effective therapeutic effect in treatment of ulcerated haemangiomas and it appears to be a valuable and effective treatment option for infantile haemangiomas beyond the proliferative phase, and esthetically disfiguring haemangiomas.

Assessment and comparison of fecal continence in children following primary posterior sagittal anorectoplasty and abdominoperineal pull through for anorectal anomaly using clinical scoring and MRI.

INTRODUCTION We aimed to compare and contrast these main surgical procedures for ARM in terms of structural outcome through pelvic MRI & functional outcome through Kellys scoring. MATERIAL AND METHOD A prospective study was conducted from August 2011 to July 2013 including all the cases of ARM managed in single stage (operated by one surgeon, first author) since 1995 that came for follow up at age of 3 years or more. Patients were divided in three groups: Group 8.A, PSARP (60 patients); Group B, Abdomino-PSARP (40 patients); and Group C, APPT (40 patients). The functional assessment of anal continence was carried out at the age of 3years or more using Kellys method. Structural assessment was done by 1.5-T Magnetic Resonance Imaging (MRI). RESULTS In 24 patients with rectobulbar fistula, 71.5% of Group A patients had good continence. In 50 patients with rectoprostatic fistula, 73.7% of Group A, 70% of group B and only 36% of Group C had good outcomes. Better development of each muscle was associated with better outcomes in terms of anal continence (P=0.001). CONCLUSION There is better outcome with PSARP and Abdomino-PSARP in patients with rectobulbar and rectoprostatic fistula. MRI is a valuable modality for postoperative structural analysis of patients with ARM and is also useful for predicting the long term functional outcome of these cases.

Management of anorectal malformation without ligation of fistula: An approach preventing posterior urethral diverticula

OBJECTIVE The posterior urethral diverticulum is a common urologic complication requiring reoperations in anorectal malformation cases (ARM). We present a series of 24 cases of male ARM managed without ligation of fistula. MATERIAL AND METHODS A prospective study was conducted between July 2010 and June 2012 including male neonates with ARM, where rectobladder neck and rectoprostatic fistula were approached by the abdominal route. The fistulous tract was dissected to the distal-most possible length and was excised flush with the urethra without its ligation. A per-urethral catheter was placed in situ. A record was made of any features of urinary leak and a micturating cystourethrogram was performed at the 1-year of follow-up. RESULTS Twenty-four cases of ARM, 16 with rectobladder neck fistula and eight cases with rectoprostatic fistula were included. Of these, 12 had single-stage primary abdominoperineal pull-through and 10 were managed by primary posterior sagittal anorectoplasty. Two cases with colostomy during the neonatal period were managed by laparoscopic assisted anorectoplasty at 6 months. None of the cases had a urinary leak during the postoperative period. All had a normal micturating cystourethrogram at 1 year. CONCLUSIONS The approach of dividing fistula without ligation may prevent posterior urethral diverticula, but larger long-term follow-up studies are needed.

Respiratory Distress Secondary to Rhabdomyosarcoma of the Tongue and Co-existent Choanal Atresia.

Whilst rhabdomyosarcoma (RMS) is the third most common solid tumour in children, congenital RMS of the tongue is extremely rare and usually present as painless progressive mass since birth (Dagher and Helman in Oncologist 4:34–44, 1999; Childs and Goudy in Int J Pediatr Otorhinolaryngol 5:126–128, 2010). In neonates, presentation with respiratory distress is unexpected as neonates are preferential nasal breathers and restricted oral breathing due to tumour usually poses no problem. We herein report a case of rhabdomyosarcoma of the tongue with co-existent unilateral choanal atresia, presenting with respiratory distress. The baby developed upper respiratory tract infection following which developed severe respiratory distress. Airway symptoms were precipitated as there was combined obstruction of both the nostrils due to infection or adenoid enlargement and unilateral chonal atresia. Treatment of respiratory distress in the presence of RMS and bilateral nasal pathology must first prioritise the security of the airway, before taking a multi-factorial approach to the therapy of the lingual mass (Childs and Goudy in Int J Pediatr Otorhinolaryngol 5:126–128, 2010). This case illustrates the importance of vigilance with respect to co-existent nasal pathology, in order to avoid the occurrence of complete airway obstruction. We therefore feel that any diagnosis of lingual RMS should warrant a formal examination of both nasal cavities.

Single stage management of a unique variant of congenital pouch colon with triplet fistula and normal anus

Congenital pouch colon (CPC) in the female patient presents with highly variable and anomalous anatomy. We herein report the first case of CPC with uterus didelphys having normal anal opening, H-type vestibular fistula, two other fistulous communications between pouch colon and two vagina managed in a single stage with excellent postoperative outcome.

Foreign bodies in digestive tract of children: a tertiary care hospital experience.

Introduction: Foreign bodies in the digestive tract are an important cause of morbidity and mortality in paediatric age group and pose diagnostic and therapeutic challenges We performed this study to evaluate our experience of foreign bodies of digestive tract in children over a five year period in a tertiary referral center. Patients and Method: A retrospective study was conducted over a 5-year period between April 2009 and March 2013. All patients who were managed for foreign body in digestive tract up to 12 years of age were included and analysis was performed from case record of patients. Results: Total 97 patients with FB in digestive tract were included in the study FB was most commonly lodged in Upper esophagus in 61 patients, middle esophagus in 12 cases lower third of esophagus in 14 cases and beyond gastro-oesophageal junction in 10 cases. The most common clinical presentations were dull pain. Coins were the most common type of foreign body in the esophagus accounting for 71.1% of patients. Plain neck and chest x-rays antero-posterior and lateral view was diagnostic in all cases, with all FB being opaque. Rigid oesophagoscopy and removal under general anesthesia was the main treatment modality performed in 63 (65.8%) and Magill forceps extraction was done in 20 cases. Conclusion: Foreign bodies in digestive tract of children are a common problem with diverse presentation. Disc battery ingestion is prone for complication and expedient removal is required & associated conditions can pose risk for lodgment of foreign bodies.

Role of intralesional bleomycin and intralesional triamcinolone therapy in residual haemangioma following propranolol

With the emergence of propranolol as the first choice of treatment for problematic infantile haemangioma at many centres, the number of patients with a partial or non-response to propranolol has also been growing. This study investigated the role of intralesional bleomycin and triamcinolone in patients with residual disease following propranolol therapy for infantile haemangioma. Sixty-seven patients with residual haemangioma were assigned randomly to receive either intralesional bleomycin (group A, n=36) or intralesional triamcinolone (group B, n=31). The response to treatment and adverse effects were assessed in both groups. All patients received at least four doses and a maximum of six doses of the assigned drug. In group A (mean follow-up 9.38months), 47.2% had an excellent response and 44.4% a good response. In group B (mean follow-up 7.42months), 25.8% had an excellent response and 48.4% a good response. There was no difference in overall response between the groups (P=0.074). Among patients who were initially non-responders to propranolol, bleomycin showed a better response than triamcinolone (P=0.037). This may be due to an overlap in the mechanism of action of propranolol and triamcinolone. Thus, intralesional bleomycin should be preferred in patients with no initial response to propranolol therapy, while bleomycin or triamcinolone can be used in patients with a partial response to propranolol therapy, as they have equal efficacy.

Simultaneous single-staged repair of anorectal malformation with tracheoesophageal fistula: Lessons learned

Introduction: Anorectal malformation (ARM) associated esophageal atresia (EA) with tracheoesophageal fistula (TEF) spawns special therapeutic propositions. The outcome of these patients banks on numerous factors. We performed this study with an aim to compare the outcome of single-staged simultaneous primary repair of both anomalies versus staged repair of these disorders. Materials and Methods: Retrospective review of cases with ARM and associated EA with TEF managed over a period of 5 years from July 2010 to June 2015 after ethical approval was undertaken. Patients were split into two groups based on whether they underwent staged repair (Group A) or single-staged simultaneous primary repair of ARM with TEF (Group B). Patients records were analyzed for demography, weight, gestational age, associated anomaly, preoperative and postoperative sepsis screen results, early and late postoperative complications (at least up to 1 year of age). Patients Kelly score for continence at the age of 3 years or more was compared. Observation: A total of 28 were included in the study. Among these, 17 were managed with staged procedure for ARM (Group A), whereas 11 underwent simultaneous single-stage repair of ARM with TEF (Group B). No difference in continence score was observed in outcome between the two groups (P = 0.96). Overall mortality in Group A at 1-year follow-up was 52.9% and in Group B was 43.4%. Conclusion: The simultaneous single-staged primary repairs result in better long-term outcome in our setup.

Systematic Imaging Module in Complete Hindgut Duplication

Complete hind gut and anal canal duplication is a rare entity, usually remaining asymptomatic till the disease comes to light due to associated anomalies or due to cosmetic reasons. Classical imaging consisting of barium enema examination served a limited role, in terms of depicting the length of gut segment involved. Technical advances in magnetic resonance imaging (MRI) with three-dimensional (3D) reformations cannot only solve the above purpose but further evaluate key points needed for surgical planning. The present technical report lays out a systematic module for evaluation of various aspects of complete hindgut duplication, critical for management. The role of 3D MRI is emphasized upon, for evaluation of pelvic floor and anorectum, even in infants with a distorted anatomy.

Subglosso-palatal membrane a rare cause of neonatal respiratory distress: A case report

Subglosso-palatal membrane is a rare cause of acute respiratory distress in newborn. Contrary to the earlier reported cases in which immediate splitting or excision of membrane has been described as measure to relief respiratory distress, we managed our case successfully without any emergency surgical procedure.